CLN3 sirna

CLN3 siRNA (Human)

Cat. Num. AAA8230621

• Gene Names: CLN3; BTS; JNCL
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: CLN3; CLN3 siRNA (Human); BTS; Battenin; Batten disease protein; Protein CLN3; CLN3 sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: CLN3 siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
• Purity/Purification: > 97%
• Form/Format: Lyophilized powder
• Sequence Length: 438

• Applicable Applications for CLN3 sirna: RNA Interference (RNAi)
• Quality Control: Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
• Directions for Use: We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
• Components: We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human CLN3 gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
• Preparation and Storage: Shipped at 4 degree C. Store at -20 degree C for one year.

Related Product Information for CLN3 sirna

siRNA to inhibit CLN3 expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 4502889
• NCBI GeneID: 1201
• NCBI Accession #: NP_000077.1
• NCBI GenBank Nucleotide #: NM_000086.2
• UniProt Accession #: Q13286; O00668; O95089; Q549S9; Q9UP09; Q9UP11; Q9UP12; Q9UP13; Q9UP14; B2R7J1; B4DXL3
• Molecular Weight: 45,155 Da
• NCBI Official Full Name: battenin isoform a
• NCBI Official Synonym Full Names: ceroid-lipofuscinosis, neuronal 3
• NCBI Official Symbol: CLN3
• NCBI Official Synonym Symbols: BTS; JNCL
• NCBI Protein Information: battenin
• UniProt Protein Name: Battenin
• Protein Family: Protein
• UniProt Gene Name: CLN3
• UniProt Synonym Gene Names: BTS
• UniProt Entry Name: CLN3_HUMAN

NCBI Description

This gene encodes a protein that is involved in lysosomal function. Mutations in this, as well as other neuronal ceroid-lipofuscinosis (CLN) genes, cause neurodegenerative diseases commonly known as Batten disease or collectively known as neuronal ceroid lipofuscinoses (NCLs). Many alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2008]

Uniprot Description

CLN3: Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes. Defects in CLN3 are the cause of neuronal ceroid lipofuscinosis type 3 (CLN3); also known as Batten disease. A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The hallmark of CLN3 is the ultrastructural pattern of lipopigment with a fingerprint profile, which can have 3 different appearances: pure within a lysosomal residual body; in conjunction with curvilinear or rectilinear profiles; and as a small component within large membrane-bound lysosomal vacuoles. The combination of fingerprint profiles within lysosomal vacuoles is a regular feature of blood lymphocytes from patients with CLN3. Belongs to the battenin family. 5 isoforms of the human protein are produced by alternative splicing.

Protein type: Autophagy; Vesicle; Chaperone; Mitochondrial; Apoptosis; Membrane protein, integral; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 16p12.1

Cellular Component: Golgi apparatus; neuron projection; Golgi stack; mitochondrion; lysosome; lysosomal membrane; endoplasmic reticulum; early endosome; integral to membrane; autophagic vacuole; caveola; trans-Golgi network; lipid raft; Golgi membrane; synaptic vesicle; cytoplasm; late endosome; plasma membrane; integral to endoplasmic reticulum membrane; nucleus

Molecular Function: protein binding; unfolded protein binding

Biological Process: sphingomyelin metabolic process; autophagic vacuole fusion; macroautophagy; glucosylceramide metabolic process; negative regulation of macroautophagy; amyloid precursor protein catabolic process; neurotransmitter metabolic process; vesicle transport along microtubule; protein catabolic process; negative regulation of neuron apoptosis; neuromuscular process controlling balance; associative learning; negative regulation of proteolysis; amino acid metabolic process; regulation of action potential; receptor-mediated endocytosis; cytosolic calcium ion homeostasis; vacuolar transport; arginine transport; globoside metabolic process; galactosylceramide metabolic process; lysosome organization and biogenesis; ionotropic glutamate receptor signaling pathway; negative regulation of catalytic activity; lysosomal lumen acidification; protein processing; ceramide metabolic process; negative regulation of apoptosis

Disease: Ceroid Lipofuscinosis, Neuronal, 3